The perfect pathogen has arrived, millions are at risk and Planet Earth will never be the same. The advent of widespread mad- cow disease--and the corresponding human epidemic of Creutzfeldt-Jakob disease--has arrived. Naturally, the US and the billion-dollar beef industry continue to deny mad cows exist in America, but our government's own data prove they do.
It's a Mad, Mad, Mad, Mad World

Every so often, a plague comes along with the power to shape nations. Such a plague is mad cow disease, or bovine spongiform encephalopathy (BSE), recognized as an expanding epidemic worldwide. Tests in Europe, where most countries routinely fed recycled cattle corpses to millions of live cattle until the crisis broke, have revealed numerous cases in addition to the 177,000 confirmed in Britain, which has incinerated nearly five million cows as a result of the crisis. The disease vector--tainted cattle feed containing the ground-up remains of cows harboring infectious protein prions--has been shipped all over the Third World, a million tons to Asia alone.

Nobody knows how many people have contracted new-variant Creutzfeldt-Jakob disease (nvCJD) through contaminated beef and bovine byproducts. Not only meat, but many processed foods, drugs, vaccines, surgical instruments, dietary supplements and even cosmetics are at risk of carrying this plague, spread through the forced cannibalism of millions of bovines. In Britain and beyond, maternal transmission of nvCJD presages generations of victims.

The US Department of Agriculture, which annually allowed a billion pounds of "rendered" protein to be fed to cattle before instituting a weak ban in 1997, adamantly denies mad cows exist in America, but leading government and private researchers, several key studies and even statistical probability contradict these assertions. The agency itself has admitted that "the potential risk of amplification of the BSE agent is much greater in the United States" than in Britain. With 100 million head of cattle--and the highest per-capita consumption of beef outside of Argentina--America could soon be revealed as the planet's biggest mad-cow sanctuary.

Meanwhile, chronic wasting disease (CWD), a similar condition which affects deer and elk, is epidemic in parts of the Southwest, and scrapie, the mad-sheep analogue suspected of infecting British cattle with BSE, has spread unchecked to 45 states. In addition to maternal transmission, CWD, scrapie and BSE are also known to be transmitted horizontally from one live animal to the next [The data showing BSE horizontal transmission is less clear--BSE coordinator], perpetuating infection even in the absence of external agents.

Infectious prions represent truth stranger than science fiction. Virtually indestructible, they represent an entirely new class of pathogen--and medicine's worst nightmare. With unique abilities to survive temperatures upward of 1,100 degree's F, jump species barriers, evade the immune system and replicate themselves in victims whose very bodies remain infectious, these rogue proteins are sowing widespread devastation among animals and humans. Even the AIDS virus is neutralized by boiling water; prions aren't even alive.

The long-term implications for the planet and its human and animal inhabitants are staggering. The list of vehicles which may harbor this hidden killer runs like a shopping list of common products. Not even vegetarians are immune: white sugar is bleached with cow bones, and McDonald's French fries, advertised as prepared in "pure vegetable oil," are seasoned--like many products with "natural flavors"--with beef fat.

The spread of BSE has given birth to the emerging industry of prion diagnostics, which is rapidly growing to fill a demand for tests. Although post-mortem tests for BSE are now widely used in Europe, tests for BSE and CJD on live subjects are not yet available.

CJD and nvCJD

CJD--the human form of mad-cow disease--and BSE are both transmissible spongiform encephalopathies, or TSEs, which literally cause spongy holes in the brain. Such "prion diseases" sporadically afflict humans, bovines and many other animals at the rate of one in a million victims. CJD, which primarily affects the elderly, can incubate for decades before leading to loss of coordination, mental breakdown and certain death. There is currently no treatment or cure.

The 100 British victims of nvCJD--which has a shorter incubation period--have been mostly younger people between 13-40 years of age. "Health officials say they've got mad cow under control, but millions of unaware people may be infected," warned a Newsweek cover story on March 12. "[O]nce a few cattle contracted it, 20th-century farming practices guaranteed that millions more would follow. For 11 years... British exporters shipped the remains of BSE-infected cows all over the world [to] more than 80 countries."

A Different US Strain?

Over the last decade the USDA has tested 12,000 cow brains, primarily looking for the pathology seen in infected British cattle, and continues to claim that not a single BSE-infected cow has been found. The US Centers for Disease Control (CDC), which has refused to mandate CJD as a reportable disease in the face of many petitions, similarly asserts that only about 280-300 people a year die from it (about one for each million Americans), with no nvCJD detected in the US.

But what if America has been harboring a different and stealthy strain of BSE all along, with a corresponding variant of CJD, and neither were being detected by current methodology? "I don't expect the British strain of mad-cow disease to be much of a problem here," says Dr. Tom Pringle, a molecular biologist who administers the astonishingly extensive "Official Mad Cow Website" (mad-cow.org). "The main fear is that our own cattle may carry a different strain of the disease that is distinct from the British strain." TSEs are known to exist in numerous strains within a single species; sheep scrapie has at least 20 variants.

The evidence for epidemics of both BSE and CJD is persuasive: 1) In 1985 Dr. Richard Marsh, a TSE researcher at the University of Wisconsin investigating a mysterious outbreak of transmissible mink encephalopathy (TME) in that state, found that the minks' diet consisted almost exclusively of "downer" cows--animals too sick to stand. 100,000 downer cows die each year in the US. In 1994 Marsh showed that when the brains of infected cattle were fed to healthy mink, they developed TME; healthy cattle inoculated with tissues obtained from TME-infected mink duly developed BSE. These experiments showed "the presence of a previously unrecognized scrapie-like infection in cattle in the United States."

The disease was different from that seen in Britain. Significantly, rather than exhibiting overt mad-cow symptoms, the animals simply collapsed; European cattle with BSE usually act skittish and "crazy" before death. In 1990 cows in Texas experimentally inoculated with American scrapie developed BSE and became lethargic and staggered to their death, just like downer cows. Some states, such as New York, don't send downer cows for USDA testing, leaving open the possibility that BSE in thousands of suspect animals is going undetected. According to Prionics, which manufactures Europe's leading BSE test, "A study performed with Prionics-Check reveals that fallen stock... represent BSE high-risk categories."

2) The USDA describes their BSE testing program as "aggressive." However, leading scientists concur that mad cows surely exist in the US. Dr. Clarence Gibbs, a pre-eminent TSE researcher who ran the laboratory of the National Institute of Neurological Disorders and Stroke until his death on Feb. 16, and chaired a World Health Organization investigation into BSE, had no doubts about domestic infection: "Do I believe BSE is here? Of course I do." And Dr. Stanley Prusiner, who won the 1997 Nobel Prize in Medicine for his discovery of prions, echoed that contention to a congressional caucus in May 1996. A June 1996 article in Food Chemical News stated, "After more than two decades of research on prions, Stanley Prusiner of the University of California at San Francisco suggested that mad-cow disease must be present in US cows at low levels.... He said he agreed with [Marsh] who believes mad-cow disease was linked to US cows in the mid-1980s."

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